Loiacono Giulia, Cirillo Chiara, Chiarelli Francesco, Verrotti Alberto
Department of Paediatrics, University of Chieti, 66100 Chieti, Italy.
ISRN Neurol. 2011;2011:867503. doi: 10.5402/2011/867503. Epub 2011 Jul 7.
Glioneuronal tumors are an increasingly recognized cause of partial seizures that occur primarily in children and young adults. Focal epilepsy associated with glioneuronal tumors is often resistant to pharmacological treatment. The cellular mechanisms underlying the epileptogenicity of glioneuronal tumors remain largely unknown. The involved mechanisms are certain to be multifactorial and depend on specific tumor histology, integrity of the blood-brain barrier, characteristics of the peritumoral environment, circuit abnormalities, or cellular and molecular defects. Glioneuronal tumors presenting with epilepsy were observed to have relatively benign biological behavior. The completeness of the tumor resection is of paramount importance in avoiding tumor progression and malignant transformation, which are rare in cases of epileptogenic glioneuronal tumors. An evolving understanding of the various mechanisms of tumor-related epileptogenicity may also lead to a more defined surgical objective and effective therapeutic strategies, including antiepileptogenic treatments, to prevent epilepsy in at-risk patients.
神经胶质神经元肿瘤是导致部分性癫痫发作的一个日益被认识到的原因，主要发生在儿童和年轻人中。与神经胶质神经元肿瘤相关的局灶性癫痫通常对药物治疗有抵抗性。神经胶质神经元肿瘤致痫性的细胞机制在很大程度上仍然未知。所涉及的机制肯定是多因素的，并且取决于特定的肿瘤组织学、血脑屏障的完整性、肿瘤周围环境的特征、回路异常或细胞和分子缺陷。观察到伴有癫痫发作的神经胶质神经元肿瘤具有相对良性的生物学行为。肿瘤切除的完整性对于避免肿瘤进展和恶性转化至关重要，这在致痫性神经胶质神经元肿瘤病例中很少见。对肿瘤相关致痫性的各种机制的不断深入理解也可能导致更明确的手术目标和有效的治疗策略，包括抗癫痫治疗，以预防高危患者发生癫痫。
